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The natural history of otosclerosis. A correlation of the volume and activity of the otosclerotic lesion with age.

Two-hundred and fifty years after Valsalva's discovery that fixation of the stapes could be a cause of deafness, there is still a lack of knowledge about the etiology and pathogenesis of otosclerosis. The purpose of this study was to obtain more information about the natural history of this disease by correlating the volumes and activity of otosclerotic lesions within the temporal bone to the patient's age and sex. Fifty temporal bones from 33 patients (19 females and 14 males) with otosclerotic lesions were reviewed microscopically. The degrees of activity of these foci were assessed and, using a computer based technique known as "digitizing", the volumes of each otosclerotic lesion were calculated. The results of this study indicate that there is no statistical difference in volumes between males and females. From the results obtained, it is suggested that there appear to be two growth patterns of the otosclerotic lesion: one pattern grows for a very short period and then becomes dormant or inactive. The other type of otosclerosis shows a continuing growth and progression throughout life.

Adolescent↗

Otosclerosis and vestibular dysfunction.

Vestibular dysfunction associated with otosclerosis has been observed for many years. In a recent clinical study of patients exhibiting progressive sensorineural hearing loss of unknown origin, it was demonstrated that approximately 50% of the cases had cochlear otosclerosis/otospongiosis as the most probable cause for the hypacusis. Of these, approximatley 55% had vestibular symptoms. In the present study, the effect of sodium fluoride therapy in this group of patients is analyzed. It will be demonstrated that such therapy is beneficial not only in controlling the progressive sensorineural hearing loss but also the symptoms of vestibular dysfunction in patients with cochlear otosclerosis (otospongiosis).

Adult↗

Otosclerosis.

The following is an English translation of an article written by Adam Politzer in 1913. Otologists of that day, concerned with the more urgent and widespread problems of ear infections and their often-fatal consequences, devoted little time to the study of otosclerosis; many dismissed it as yet another post infectious complication of the mucosa or periosteum of the middle ear. Politzer, using the preeminent research tool of the New Vienna School, the autopsy, was the first to establish our current view that otosclerosis is a primary disease of the labyrinthine capsule. The section on clinical diagnosis is valid and useful today. Except for the use of iodine, believed to slow the spread of the illness, Politzer considered all medical and surgical treatment employed at that time, including the primitive stapedectomy, to be futile.

Ear, Inner↗

[Scarpa's ganglion with increasing age, in Menière's disease and otosclerosis (author's transl)].

A quantitative study of primary vestibular neurons was performed in temporal bones of individuals at different ages. The mean numbers of counts decreased beginning in the age group between 50 and 60 years. Neuronal populations from cases with Menière disease and otosclerosis were compared to the respective "normal" values. Endolymphatic hydrops could be excluded as the cause of degeneration of Scarpa's ganglion in Menière' disease. In cases of otosclerosis reduced neuronal counts were found if lesions were large enough to include the cribrose areas.

Adolescent↗

[Pathogenetic aspects of otosclerosis].

Basic otosclerosis pathogenetic factors are reviewed with a special emphasis on heredity. The inheritance in otosclerosis is autosomal dominant with incomplete gene expression. The involvement of HLA antigens is detailed. Autoimmune origin of the disease is confirmed morphologically and immunologically in perilymph and serum tests. There are infiltrates with lymphocytes, plasmatic and mast cells in basis stapedial mucoperiostal tissue. Perilymph contained significantly elevated levels of IgG and IgM, of antibodies to native DNA and type II collagen, especially in patients with active otosclerotic foci. Validation of relevant etiopathogenetic mechanism is provided basing on first-hand experience and literature data.

Autoimmune Diseases↗

The effect on tinnitus of stapes surgery for otosclerosis.

Among 42 Japanese patients with otosclerosis who underwent stapes surgery, 22 patients including 10 received total stapedectomy, 6 partial stapedectomy and 6 stapedectomy, were investigated on preoperative and postoperative tinnitus by sending questionnaire. The tinnitus was evaluated by "tinnitus score" advocated by the Japan Audiological Society, and the changes of the scores were analyzed according to pre- and postoperative hearing change, type of stapes surgery and pitches of preoperative tinnitus. In these 22 patients, tinnitus score was improved in 68%, unchanged in 27% or worsened in 5%. Tinnitus was completely disappeared in 6 patients or 27.3%. Tinnitus score became more markedly improved in the patients received partial stapedectomy or stapedotomy than those who received total stapedectomy. Tinnitus score was more likely improved in the patients who had a low pitched tinnitus and presumably had no cochlear otosclerosis preoperatively.

Adult↗

[CT (high-resolution) in 60 cases of stapediovestibular otosclerosis with surgical indications].

Sixty patients with stapediovestibular otosclerosis (OEV) were studied by CT-scan (target program). These patients had stapedectomy indication because they had a conductive hearing loss in which one had an A/B gap of > 25 dB. The leading principle of this study was how to know the main indications of the CT-scan which makes it possible for morphologic and densitometric evaluation of the extension and probable activity of the stapedial, fenestral and fissural labyrinthine otosclerotic foci. A correlation between the CT pattern of otosclerosis and the pure or mixed hearing loss had been tried. The conclusion is that CT should be a routine examination of OEV clinical study.

Adult↗

[Otosclerosis. Immunoendocrine and infective aspects].

The etiopathogenesis of otosclerosis is yet scarcely known. In this paper we focus on the immunoendocrine and infective aspects of the disorder. According to recent investigations the role played by cytokines and calcitonin's receptors appears to be crucial in the pathogenesis of otosclerosis. In addition, the ultrastructural and immunohistochemical evidence of virus specific antigens around the active otosclerotic focus suggest that virus can trigger and maintain an inflammatory disease.

Hormones↗

[The radiological pattern of cochlear otosclerosis. A CT study of 14 patients].

In this review of cochlear otosclerosis 14 cases were studied by CT scan aiming to establish a densitometric pattern of the capsular foci and relating it to the hearing and vestibule dysfunctions. Severe demineralization with characteristics of probable activity (increased lucency of 30-40%) was demonstrated in the capsular foci. These were mainly cochlear with endosteal involvement (93%): large (64%) or discrete (29%). Cochlear otosclerosis was widespread in 64% of the patients, with coexisting foci in the semicircular canals (38%), vestibule aqueduct (43%) and internal auditory canal (43%). The antefenestral component with stapes involvement was 85%, mostly of the anterior polar and crural varieties (64%) and signs of activity. In 2 patients there was a conductive hearing loss in the tonal audiometry, pure or combined; in 2 others there was only a pure perceptive hypoacusis of type IV. A direct relationship was noted (64% of cases) between the most serious hypoacusis (type III and IV) and the endosteal extension of the cochlear foci. Vertigo occurred in 36% of the patients and was attributed to the posterior labyrinth foci.

Adolescent↗

Far-advanced otosclerosis.

Far-advanced otosclerosis (FAO) is an uncommon diagnosis. Hearing levels in patients with FAO may range from profound loss, by air conduction and fragmentary bone conduction thresholds, to no measurable air or bone conduction thresholds. Thus, FAO may be difficult to distinguish from a sensorineural hearing loss. This report presents the results of surgery in 73 ears with FAO, 77 percent of which had improvement in air conduction thresholds of greater than 20 dB. Discrimination was improved by more than 15 percent in 54 percent of cases, and 75 percent realized improvement in use of a hearing aid. There was no evidence that success was related to preoperative hearing. The surgical results of a subgroup of 14 patients having bilateral FAO were also analyzed. For all 14, similar surgical outcomes were achieved in both the initial and the contralateral ear, with six successes bilaterally and eight failures bilaterally. Although far advanced otosclerosis is uncommon and difficult to diagnose, surgery is worthwhile.

Acoustic Stimulation↗

The evolution of otosclerosis surgery.

This article reviews the development of surgical methods over the centuries to improve hearing loss due to otosclerosis. Pioneers in the development of surgical techniques for otosclerosis discussed in this article include Holmgren, Lempert, Rosen, and Shea. Also discussed are the personal experiences of the author, witness to many of this century's technologic developments and founder of the House Ear Institute in Los Angeles, California.

General Surgery↗

Histopathology of otosclerosis.

This histopathologic review illustrates that otosclerosis is a very pleomorphic bone dyscrasia. It may fix the stapes by a tiny bridge of abnormal bone or may totally obliterate it. It may spread to the cochlea to produce either a sensorineural loss of varying degree or deafness. Lesions may be sclerotic, spongiotic, or fibrous but usually comprise all these histologic types. This diversity of size and anatomic distribution coincides with the variable types of hearing loss, from a mild conductive deficit to a total loss. In spite of more than a century of investigation of otosclerosis, an etiologic agent other than heredity has not been established.

Ear Ossicles↗

[Mastoid pneumatization in otosclerosis].

With the use of planimetry, and ears lateral projection X-ray pictures, the size of temporal bones pneumatization surfaces was measured 88 ears with otosclerosis and 88 healthy ears were examined. The degree of pneumatization in the two groups was compared. Statistically significantly highly pneumatization was found in the group of ears with otosclerosis.

Adolescent↗

Surgery in elderly patients with otosclerosis.

Hearing results and complications of surgery were studied in 42 patients with otosclerosis (46 ears operated on) who were over the age of 60 years at the time of stapedectomy. The mean follow-up period was 8 years. The results were compared with those obtained in 275 patients (330 ears operated on) younger than 60 years of age undergoing stapes surgery during the same time period. Large fenestra stapedectomy with fascia seal to the oval window was used in all cases. Hearing results as judged by postoperative air-bone gaps were as good in the older age group as in the younger patients. In contrast to some earlier reports, complications of surgery such as postoperative sensorineural hearing loss occurred not more frequently among elderly patients than in younger patients. It is concluded that stapes surgery should be offered to elderly patients with the same indications as in younger patients with otosclerosis.

Age Factors↗

[Cytogenetic study in otosclerosis].

In 50 patients (32 females and 18 males) with otosclerosis who underwent stapedectomy, a cytogenetic study of chromosomal abnormalities was made. No description of G banding in patients with otosclerosis was found in the literature. No chromosomal abnormalities were found in the karyotype using this technique.

Chromosome Aberrations↗

[High-resolution CT of otosclerosis].

High-resolution CT (HRCT) scans of thirty-two patients (60 ears) with the clinical diagnosis of fenestral otosclerosis were evaluated retrospectively. HRCT was performed with 1-mm-thick targeted sections and 1-mm (36 ears) or 0.5-mm (10 ears) intervals in the semiaxial projection. Seven patients (14 ears) underwent helical scanning with a 1-mm slice thickness and 1-mm/sec table speed. Forty-five ears (75%) were found to have one or more otospongiotic or otosclerotic foci on HRCT. In most instances (30 ears), the otospongiotic foci were found in the region of the fissula ante fenestram. No significant correlations between CT findings and air conduction threshold were observed. We found a significant relationship between lesions of the labrinthine capsule and sensorineural hearing loss. We conclude that HRCT is a valuable modality for diagnosing otosclerosis, especially when otospongiotic focus is detected.

Adolescent↗

Word recognition score changes after stapedectomy for far advanced otosclerosis.

OBJECTIVE: This study aimed to examine word recognition score (WRS) changes after stapedectomy for far-advanced otosclerosis (FAO). The WRS changes were examined to determine whether they were consistent with acclimatization or recovery from auditory deprivation changes that have been seen after the restoration of sound by amplification. STUDY DESIGN: Retrospective. SETTING: Private otology practice. PATIENTS: A total of 24 patients were selected by including all the case in which a stapedectomy was performed within the past 10 years to improve the hearing of a severe or profoundly hearing-impaired patient with otosclerosis. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURE: Changes in WRSs. RESULTS: One month after surgery, the mean WRS had improved 16.5%. The WRSs continued to improve an additional 12% or more for 17 (71%) of 24 patients within 2 years after their initial postoperative hearing test. The mean WRS improvement within 2 years of the initial postoperative test was 16.2%. CONCLUSIONS: Initial WRS changes were attributed to hearing thresholds no longer being at or beyond audiometric limits. Additional WRS changes were consistent with reports of acclimatization or recovery from auditory deprivation that have been seen after hearing aid use. The authors believe these additional WRS changes illustrate that at least some improvement in WRSs from acclimatization or auditory recovery may be fairly common after the restoration of sound. Finally, the authors believe the overall WRS improvement (32.7%) should be taken into account when considering stapedectomy for patients with FAO.

Audiometry, Pure-Tone↗

[Calcitonin and otosclerosis: a preliminary clinical note].

Otosclerosis is a disorder characterized by alternating periods of bone resorption and formation as a result of a disturbance in the enzymatic balance of the otic capsule and stapedial footplate. In some cases, calcitonin administration improved the metabolic abnormalities associated with the disease. This hormone inhibits bone resorption produced by osteoclasts and facilitates osteoblastic accretion. The authors describe the results obtained in a patient with aggressive otosclerosis who experienced an improvement in hearing and tinnitus after treatment with salmon calcitonin.

Administration, Intranasal↗